中国南方儿童急性播散性脑脊髓炎回顾性研究

Acute disseminated encephalomyelitis among children in the south of China

目的探讨儿童急性播散性脑脊髓炎(ADEM)临床特征。方法回顾性分析36例ADEM患儿的临床表现(包括影像学特点及实验室检查)、治疗及预后情况。结果 (1)临床表现:36例ADEM患儿,其中有22例(61.1%)发生在冬季或春季,22例(61.1%)有上呼吸道感染。临床表现为运动缺陷最多,有23例(63.9%),其次是意识改变14例(38.9%)。2例患儿EB病毒抗原DNA阳性,5例患儿肺炎支原体IgM阳性;28例行脑脊液检查,其中15例(53.6%)发现异常,表现为脑脊液细胞增多或蛋白增多;31例行头MRI检查,异常27例,其中皮质下白质受累20例,室周白质受累9例,累及深部灰质6例,累及脑干4例;6例行脊髓MRI检查,均发现片状异常。(2)治疗及转归:27例患儿接受糖皮质激素治疗,25例接受免疫球蛋白治疗。出院时31例恢复正常,3例留有轻微的后遗症。结论感染是ADEM的主要诱因。MRI能显示病变范围,可为临床诊断提供重要信息。ADEM患者预后较好。

Objective To investigate the clinical features in a cohort of children with acute disseminated encephalomyelitis （ADEM）. Methods The clinical features of 36 patients with ADEM were analyzed, including neuroimaging and laboratory features, treatment and prognosis. Results （1） Clinical manifestations： Thirtysix cases were identified. Twenty-two patients （61.1%） presented in either winter or spring. Twenty-two （61.1%） had a recent upper respiratory tract illness. Patients presented most often with motor deficits in 23 children （63.9%） and secondly with altered consciousness in 14 （38.9%）. A definite microbiologic diagnosis was established only in 2 child with Epstein-Barr virus disease and 5 patients had elevated immunoglobulin M titers to Mycoplasma pneumoniae. Cerebrospinal fluid （CSF） examination was undertaken in 28 patients and was abnormal in 15, either pleocytosis or elevated protein. Brain magnetic resonance imaging was carried out in 31 patients and abnormal in 27. There were identified lesions in subcortical white matter in 20 patients, in periventricular white matter in 9, in deep gray matter in 6, and in brainstem in 4 of patients. Six patients had abnormal spinal MRI. （2） Treatment and prognosis： Twenty-seven patients were treated with corticosteroids, and twenty-five were treated with intravenous immunoglobulins. Clinical normalization occurred in 31, and 3 displayed mild sequela signs. Conclusions Epidemiologic evidence from this study suggest infectious cause for ADEM. MRI can demonstrate the evidence for the diagnosis. Prognosis is generally good.